A rare case of Camptocormia (bent spine syndrome)

Clinical Cases 29.08.2011
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Section: Musculoskeletal system
Case Type: Clinical Cases
Patient: 76 years, female
Authors: Yallapa, Sachin; Bashir, Omar; Sheik, Mujeeb.
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Details
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AI Report

Clinical History

The patient presented with discomfort in lower back after prolonged standing or walking for 3 years, progressive difficulty to maintain an erect posture. Difficulty to stand up straight from stooped posture and difficulty walking. No history of any neurology in upper or lower limbs. No family history of any neuromuscular disorder.

Imaging Findings

Disc degeneration throughout the lumbar region with a varying degree of disc space narrowing and disc signal loss. There is some bulging of these discs but no evidence of disc protrusion or root compression. The bony canal and the lateral root canals are adequate. Bone marrow signal is unremarkable. There is no intradural abnormality.
Significantly, gross fatty replacement of the erector spinae musculature was noted throughout the lumbar spine.

Discussion

Discussion:
Camptocormia (bent spine syndrome) refers to an abnormal posture with marked flexion of the thoraco-lumbar spine which increases during walking and resolves in the supine position. Originally attributed to psychogenic disorders (war hysteria), this symptom is an increasingly recognised feature of parkinsonian and dystonic disorders, but it may also be caused by neuromuscular disorders [3, 1] as well as paraneoplastic syndrome [4], inclusion body myositis [5] drug-induced camptocormia [4] and Grave’s disease [7].
Camptocormia is a syndrome confined to the extensor muscles of the spine. There are usually no other motor or sensory symptoms. Lumbar radicular nerves are unaffected. However, some patients may suffer mild discomfort in the lower back. Moreover, some authors suggest that symptoms may not occur unless there is also hip flexion contracture [2].
Radiological analysis of the thoraco-lumbar spine by MRI or CT can reveal paraspinal muscle atrophy heterogeneous in appearance, with progressive replacement of paraspinal muscles with fat. Electromyography findings are not uniform. Both myogenic and neurogenic features of the disease have been reported [2].

Some pharmacologic treatments attempted so far include anticholinergics, amantadine, dopamine agonists, muscle relaxants, and tetrabenazine, but these have not improved the posture [1]. There is some evidence of bilateral pallidal stimulation providing functional benefit for the severely disabling condition. Physiotherapy with classical and leather arthesis has also been tried in few cases.
However, most cases have failed to respond fully to any treatment. No treatment modality has proved to be effective so far.

Differential Diagnosis List

Camptocormia (bent spine syndrome)
Spinal deformity
Dystonic disorders

Final Diagnosis

Camptocormia (bent spine syndrome)

Liscense

Figures

CT scan lumbo-sacral region, sagittal views

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CT scan lumbo-sacral region, sagittal views
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CT scan lumbo-sacral region, sagittal views

CT lumbar spine axial views

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CT lumbar spine axial views
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CT lumbar spine axial views